Variant and Specific Forms of Autoimmune Cholestatic Liver Diseases

Dalekos, Georgios N.; Gatselis, Nikolaos

doi:10.1007/s00005-019-00550-9

Cited by 13 publications

(30 citation statements)

References 128 publications

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“…The presence of isolated AMA positivity in the absence of clinically apparent liver disease or abnormal liver function tests is seen in up to 0.5% of otherwise healthy individuals. This finding may be a hallmark of preclinical disease, and up to 17% will develop PBC over 5 years 3 , 10 . Other autoantibodies are often identified in PBC patients, particularly anti-nuclear antibodies (ANA).…”

Section: Primary Biliary Cholangitismentioning

confidence: 99%

“…In the cirrhotic stage of the disease, radiological HCC surveillance is therefore mandatory 10 . Patients with PBC, particularly women, have a higher likelihood of other concomitant autoimmune disorders, and up to 55% have an additional ongoing autoimmune process, such as autoimmune Hashimoto’s thyroiditis, Sjogren’s syndrome and Raynaud’s disease 3 , 10-12 .…”

Section: Primary Biliary Cholangitismentioning

confidence: 99%

“…AMA in a patient with raised ALP is diagnostic of PBC, after the exclusion of other intrahepatic and extrahepatic causes of cholestasis. AMA-negative PBC occurs in 5% of patients with an otherwise typical clinical, biochemical and histological profile 3 , 10 . The presence of isolated AMA positivity in the absence of clinically apparent liver disease or abnormal liver function tests is seen in up to 0.5% of otherwise healthy individuals.…”

Section: Primary Biliary Cholangitismentioning

confidence: 99%

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Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis

et al. 2021

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Summary Autoimmune cholestatic liver diseases are rare hepato-biliary disorders characterized by a progressive, inflammatory destruction of bile ducts. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the main autoimmune cholestatic liver diseases. Both may evolve into secondary biliary cirrhosis and its complications. Therapeutic options are limited and liver transplantation remains the only definitive treatment for PBC and PSC. Most PBC and PSC patients have a typical presentation, which does not require liver biopsy. However, in routine clinical practice, important variants or specific subgroups that benefit from liver biopsy for proper management may be observed. Herein, we provide a general overview of clinical and pathological characteristic of PBC and PSC, highlighting the most important features for routine diagnostic practice.

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Section: Primary Biliary Cholangitismentioning

confidence: 99%

Section: Primary Biliary Cholangitismentioning

confidence: 99%

Section: Primary Biliary Cholangitismentioning

confidence: 99%

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Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis

et al. 2021

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“…AILDs, including AIH, PBC, primary sclerosing cholangitis (PSC), and their variant forms, encompass a wide spectrum of liver diseases with different pathophysiology, clinical presentation, treatment options, and prognosis. They can co-occur either in a synchronous or metachronous fashion [ 24 , 25 ], while recognition of coexisting chronic liver diseases, including viral hepatitides, alcohol, NAFLD, and drug-induced liver disease is increasingly documented [ 26 , 27 , 28 , 29 ].…”

Section: Autoimmune Liver Diseasesmentioning

confidence: 99%

Current Trends and Characteristics of Hepatocellular Carcinoma in Patients with Autoimmune Liver Diseases

Rigopoulou

Dalekos

2021

Cancers

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Hepatocellular carcinoma (HCC), the commonest among liver cancers, is one of the leading causes of mortality among malignancies worldwide. Several reports demonstrate autoimmune liver diseases (AILDs), including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) to confer increased risk of hepatobiliary malignancies, albeit at lower frequencies compared to other liver diseases. Several parameters have been recognized as risk factors for HCC development in AIH and PBC, including demographics such as older age and male sex, clinical features, the most decisive being cirrhosis and other co-existing factors, such as alcohol consumption. Moreover, biochemical activity and treatment response have been increasingly recognized as prognostic factors for HCC development in AIH and PBC. As available treatment modalities are effective only when HCC diagnosis is established early, surveillance has been proven essential for HCC prognosis. Considering that the risk for HCC is not uniform between and within disease groups, refinement of screening strategies according to prevailing demographic, clinical, and molecular risk factors is mandated in AILDs patients, as personalized HCC risk prediction will offer significant advantage in patients at high and/or medium risk. Furthermore, future investigations should draw attention to whether modification of immunosuppression could benefit AIH patients after HCC diagnosis.

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“…Although female sex predominates, it is now recognized that AIH can affect both sexes of all age and ethnic groups [1][2][3][4]. The wide spectrum of clinical characteristics in conjunction with absence of disease-specific laboratory and histologic features, renders its diagnosis often difficult and challenging, as it could concur with other autoimmune diseases [5][6][7][8][9] and a large spectrum of acute and chronic hepatopathies such as viral hepatitis, non-alcoholic fatty liver disease (NAFLD), autoimmune cholestatic syndromes and drug-induced liver injury [1,3,[10][11][12][13][14][15][16]. In this context, data on coincidence of AIH with alcoholic liver disease (ALD) or an alternative AIH diagnosis instead of ALD is missing.…”

Section: Introductionmentioning

confidence: 99%

Alcoholic liver disease and autoimmune hepatitis: Sometimes a closer look under the surface is needed

Rigopoulou¹,

Gatselis²,

Arvaniti³

et al. 2021

European Journal of Internal Medicine

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Differential diagnosis of autoimmune hepatitis (AIH) incorporates various liver diseases, including alcoholic liver disease (ALD). We report on clinical, laboratory and outcome characteristics of AIH patients who were initially referred as ALD based on increased alcohol consumption (AIH/ALD). Methods: From 2000-2019, we retrospectively identified 12 AIH/ALD patients [9 males, age: 61 (30-73) years] in our prospective data base of 317 AIH patients. Results: AIH diagnosis was based on aminotransferases elevation in 10 patients, high IgG in 8, compatible autoantibody profile in all and typical/compatible histology in all 9 with available biopsy. There were no significant differences of baseline demographics, presentation, cirrhosis at diagnosis, response to treatment and simplified score compared to 45 age-and sex-matched AIH patients without alcohol consumption and 44 ageand sex-matched ALD patients. However, the AIH/ALD cohort was characterized by more frequent progression to cirrhosis, higher liver-related deaths and overall mortality compared to AIH, though similar to the ALD group. AST/ALT ratio>1 seems to bear a good positive (0.84) and negative predictive value (0.88) for ALD and AIH diagnosis, respectively, but cannot help in discriminating the AIH/ALD variant. Conclusions: AIH should not be forgotten in patients with alcohol use when clinical and laboratory features hint towards the diagnosis of AIH/ALD variant as this group seems to have worse outcome compared to those with AIH alone suggesting the need for closer follow-up and surveillance. Reliable autoantibody testing and cautious interpretation of liver histology appear mandatory for AIH diagnosis in these difficult to diagnose cases.

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Variant and Specific Forms of Autoimmune Cholestatic Liver Diseases

Cited by 13 publications

References 128 publications

Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis

Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis

Current Trends and Characteristics of Hepatocellular Carcinoma in Patients with Autoimmune Liver Diseases

Alcoholic liver disease and autoimmune hepatitis: Sometimes a closer look under the surface is needed

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